What drives transcranial Doppler velocity improvement in paediatric sickle cell anaemia: analysis from the Sickle Cell Clinical Research and Intervention Program (SCCRIP) longitudinal cohort study

Br J Haematol. 2021 Jul;194(2):463-468. doi: 10.1111/bjh.17620. Epub 2021 Jun 15.

Abstract

Children with sickle cell anaemia (SCA) and conditional transcranial Doppler (TCD) flow velocities (conditional: 170-199 cm/s; normal: <170 cm/s) have an increased risk of stroke. The Sickle Cell Clinical Research and Intervention Program (SCCRIP), a lifetime observational study, assessed the influence of haematological markers on TCD velocities. In children (≤16 years) with SCA (HbSS/HbSβ0 -thalassaemia) and conditional TCD velocities (n = 32), increases in haemoglobin and in fetal haemoglobin after hydroxyurea initiation were significantly associated with decreases in TCD velocities. The benefit of pharmacological intervention to increase haemoglobin and fetal haemoglobin and normalise TCD velocities was demonstrated in this real-world dataset.

Keywords: haemoglobin; hydroxyurea; sickle cell; stroke; transcranial Doppler (TCD).

Publication types

  • Clinical Trial
  • Observational Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anemia, Sickle Cell / blood
  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / drug therapy*
  • Anemia, Sickle Cell / physiopathology
  • Antisickling Agents / therapeutic use*
  • Blood Flow Velocity / drug effects
  • Child
  • Child, Preschool
  • Female
  • Hemoglobins / analysis
  • Humans
  • Hydroxyurea / therapeutic use*
  • Longitudinal Studies
  • Male
  • Stroke / blood
  • Stroke / etiology*
  • Stroke / physiopathology
  • Stroke / prevention & control
  • Ultrasonography, Doppler, Transcranial

Substances

  • Antisickling Agents
  • Hemoglobins
  • Hydroxyurea